We searched the Cochrane Library, Medline, and EMBASE, mainly with the search terms “polymyalgia rheumatica [Mesh]”, “giant cell arteritis [Mesh]”, and “temporal arteritis [Mesh]”. We largely selected articles published in English during the past 5 years, without excluding older papers that we considered to be highly relevant to the topics discussed in this Seminar. We also searched the reference lists of articles identified by this search strategy, and selected those that we judged
SeminarPolymyalgia rheumatica and giant-cell arteritis
Section snippets
Definitions and diagnostic criteria
Giant-cell arteritis mainly involves the large-sized and medium-sized arteries, especially branches of the proximal aorta. Polymyalgia rheumatica is characterised by aching and morning stiffness in the shoulder and pelvic girdles and neck. The two disorders can occur separately or together in people aged 50 years and older.
The diagnosis of polymyalgia rheumatica depends on a combination of clinical symptoms, raised acute-phase reactants, exclusion of other diseases, and response to
Epidemiology
The incidence rates of giant-cell arteritis and polymyalgia rheumatica increase progressively after 50 years of age.13 The reported rates for giant-cell arteritis are highest in northern European countries and in Minnesota (USA), which has a population of similar ethnic background, and are 20 or more per 100 000 people older than 50 years.14, 15, 16 Rates of this disease are lower in Mediterranean countries17, 18 and lowest in Arabian and Asian countries.19, 20 The lowest prevalence was
Relation between giant-cell arteritis and polymyalgia rheumatica
The clinical connections between polymyalgia rheumatica and giant-cell arteritis have suggested that they are different manifestations of the same disease process. These connections include their frequent occurrence together, the older age at onset with progressively increasing incidence rates after 50 years, similar sex ratio, substantial increase of acute-phase reactants before treatment, and rapid responsiveness to glucocorticosteroids and outcome.13 Population-based studies have shown that
Pathology and pathogenesis
In giant-cell arteritis, inflammation mainly affects the large-sized and medium-sized muscular arteries, especially the proximal aorta and its branches.13, 31 These arteries have a prominent internal elastic membrane and vasa vasorum. As the cervical arteries penetrate the dura they become thinner, have much less elastic tissue, and no vasa vasorum. Intracranial arteries are rarely associated with the vasculitic process.32
The classic histological picture of giant-cell arteritis is characterised
Polymyalgia rheumatica
Polymyalgia rheumatica is typically characterised by aching and stiffness in the morning in the neck, shoulder, and pelvic girdles. Typically the stiffness in the morning lasts 30 min or more. Shoulder pain is the presenting finding in 70–95% of patients, whereas hips and neck are less frequently involved (50–70%). The pain usually radiates distally towards the elbows and knees. It can begin in one shoulder or hip, but soon becomes bilateral. Occasionally the symptoms begin more peripherally.
Laboratory findings and imaging
Laboratory findings in both polymyalgia rheumatica and giant-cell arteritis are non-specific but indicate the inflammatory nature of these syndromes. An ESR of at least 40 mm/h has been included in all sets of criteria for the diagnosis of polymyalgia rheumatica.1, 2, 3 However, a normal ESR has been reported in 7–20% of the patients with polymyalgia rheumatica.79 The American College of Rheumatology classification criteria for giant-cell arteritis include an ESR of 50 mm/h or more.4 However,
Differential diagnosis
Several disorders can mimic polymyalgia rheumatica.97 Peripheral arthritis, particularly affecting both hands, can pose a challenge in the distinction of polymyalgia rheumatica from elderly-onset rheumatoid arthritis. Pronounced symmetrical peripheral synovitis, positive rheumatoid factor and anticyclic citrullinated peptide antibodies, and the development of joint erosions and extra-articular manifestations differentiate rheumatoid arthritis from polymyalgia rheumatica. Follow-up is sometimes
Giant-cell arteritis
Glucocorticosteroids are the treatment of choice. Adequate doses quickly suppress clinical manifestations of this disorder and prevent most further ischaemic complications. If visual loss has occurred before start of treatment, it is not usually reversed.13, 105, 106 Glucocorticosteroid therapy should be initiated as soon as the diagnosis of giant-cell arteritis is established. We recommend an initial dose of 40–60 mg per day of prednisone (or equivalent) as a single or divided dose.13 If the
Future perspectives
The development of standardised classification and diagnostic criteria would help in a comparison of studies from different centres and assist clinicians. Additional investigation is needed about the use of pulse glucocorticosteroids at the onset of treatment for giant-cell arteritis to confirm whether this regimen reduces toxic effects of glucocorticosteroids. Identification of risk factors for extended and relapsing disease might allow a more effective use of glucocorticosteroids and could
Search strategy and selection criteria
References (134)
Long-term follow-up of polymyalgia rheumatica: evidencefor synovitis
Semin Arthritis Rheum
(1984)- et al.
Efficacy of unilateral versus bilateral temporal artery biopsies for the diagnosis of giant cell arteritis
Am J Ophthalmol
(1999) - et al.
The role of unilateral temporal artery biopsy
Ophthalmology
(2003) - et al.
Initiation of glucocorticoid therapy: before or after temporal artery biopsy?
Mayo Clin Proc
(2004) - et al.
Are polymyalgia reumatica and giant cell arteritis the same disease?
Semin Arthrititis Rheum
(2004) Giant cell arteritis and polymyalgia rheumatica: two different but often overlapping conditions
Semin Arthritis Rheum
(2004)- et al.
Is giant cell arteritis and infectious disease? Biological and epidemiological evidence
Presse Med
(2004) - et al.
Genetic markers of disease. Susceptibility and severity in giant cell arteritis and polymyalgia rheumatica
Semin Arthritis Rheum
(2003) - et al.
Clinical manifestations of giant cell (temporal) arteritis
Clin Rheum Dis
(1980) - et al.
Occult giant cell arteritis: ocular manifestations
Am J Ophthalmol
(1998)
Pericardial and pleural effusion in giant cell arteritis
Am J Med
Erythrocyte sedimentation rate and C-reactive protein in the evaluation of disease activity and severity in polymyalgia rheumatica: a prospective follow-up study
Semin Arthritis Rheum
Giant cell arteritis: validity and reliability of various diagnostic criteria
Am J Ophthalmol
Polymyalgia rheumatica: a 10-year epidemiologic and clinical study
Ann Intern Med
An evaluation of criteria for polymyalgia rheumatica
Ann Rheum Dis
The American College of Rheumatology 1990 criteria for the classification of giant cell (temporal) arteritis
Arthritis Rheum
Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis
Ann Intern Med
Temporal artery biopsy for giant cell arteritis
J Rheumatol
Temporal artery biopsy: is there any value in examining biopsies at multiple levels?
J Clin Pathol
How does previous corticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis?
Ann Intern Med
Does this patient have temporal arteritis?
JAMA
Polymyalgia rheumatica and giant-cell arteritis
N Engl J Med
Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period
Arthritis Rheum
Giant cell arteritis: epidemiological clues to its pathogenesis and an update on its treatment
Rheumatology
The incidence of polymyalgia rheumatica and temporal arteritis in the county of Aust Agder, south Norway: a prospective study 1987–1994
J Rheumatol
Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in Northern Italy
Arthritis Rheum
Giant cell arteritis in Northwestern Spain. A 25-year epidemiologic study
Medicine
Epidemiology of giant cell arteritis in an Arab population: a 22-year study
Br J Ophthalmol
Clinical and epidemiologic analysis of giant cell (temporal) arteritis from a nationwide survey in 1998 in Japan: the first government-supported nationwide survey
Arthritis Rheum
On arteritis with special reference to polymyalgia arteritica
Acta Pathol Microbiol Scand (A)
The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern
Ann Intern Med
The incidence of giant cell arteritis in Jerusalem over a 25-year period: annual a seasonal fluctuations
Clin Exp Rheumatol
Mortality of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis. A population-based study over 50 years
Arthritis Rheum
Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970–1991
Arthritis Rheum
Giant cell arteritis and polymyalgia rheumatica in a region of Finland: an epidemiologic, clinical and pathologic study, 1984–1988
J Rheumatol
Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990–2001
Ann Rheum Dis
Repetitive 18-fluorodeoxyglucose positron emission tomography in isolated polymyalgia rheumatica: a prospective study in 35 patients
Rheumatology (Oxford)
Arteries of the head and neck in giant cell arteritis. A pathological study to show the pattern of arterial involvement
Arch Neurol
Giant cell arteritis: involvement of intracranial arteries
Arthritis Rheum
Illustrated histopathologic classification criteria for selected vasculitis syndromes
Arthritis Rheum
Skip lesions in temporal arteritis
Mayo Clin Proc
Small-vessel vasculitis surrounding a spared temporal artery: clinical and pathologic findings in a series of twenty-eight patients
Arthritis Rheum
Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment
Arthritis Rheum
Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection
J Rheumatol
A population-based case-control study of temporal arteritis: evidence for an association between temporal arteritis and degenerative vascular disease?
Int J Epidemiol
Giant cell arteritis and cardiovascular risk factors. A multicenter, prospective case-control study
Arthritis Rheum
Influence of traditional risk factors of atherosclerosis in the development of severe ischemic complications in giant cell arteritis
Medicine
HLA-DRB1 alleles associated with polymyalgia rheumatica in northern Italy: correlation with disease severity
Ann Rheum Dis
Medium- and large-vessel vasculitis
N Engl J Med
Vascular dendritic cells in giant cell arteritis
Ann NY Acad Sci
Cited by (649)
miR-146a and miR-146b regulate the expression of ICAM-1 in giant cell arteritis
2024, Journal of AutoimmunityPrimary Large Vessel Vasculitis: Takayasu Arteritis and Giant Cell Arteritis
2024, Neuroimaging Clinics of North AmericaCost-effectiveness analysis of the diagnosis of temporal arteritis
2024, Reumatologia ClinicaNeointimal myofibroblasts contribute to maintaining Th1/Tc1 and Th17/Tc17 inflammation in giant cell arteritis
2024, Journal of AutoimmunityConsensus statement on the processing, interpretation and reporting of temporal artery biopsy for arteritis
2023, Cardiovascular PathologyCytokine producing B-cells and their capability to polarize macrophages in giant cell arteritis
2023, Journal of Autoimmunity